help button home button Endocrine Society JCEM
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
This Article
Right arrow Submit a related Letter to the Editor
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow Request Copyright Permission
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Bergner, E. A.
Right arrow Articles by Shapiro, L. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Bergner, E. A.
Right arrow Articles by Shapiro, L. J.

Journal of Clinical Endocrinology & Metabolism, Vol 53, 221-223, Copyright © 1981 by Endocrine Society

ARTICLES

Increased cholesterol sulfate in plasma and red blood cell membranes of steroid sulfatase deficient patients

EA Bergner and LJ Shapiro

Steroid sulfatase deficiency is an inborn error of metabolism characterized during fetal life by decreased estriol production and postnatally by x-=linked ichthyosis. No consistent substrate abnormalities have been found beyond the perinatal period. Utilizing gas chromatography, we found that the cholesterol sulfate concentration was less than 350 micrograms/100 ml plasma in 9 normal adults, 2 subjects with ichthyosis vulgaris and 2 subjects with lamellar ichthyosis. Control red cell membranes had less than 300 micrograms/100 ml erythrocytes. Eight subjects (age 3 months-74 years) with steroid sulfatase deficiency had strikingly elevated cholesterol sulfate levels with means and ranges as follows: plasma - 3,300 micrograms/100 ml (2,700-4,000), red cell membranes- 7,500 (5,200-9,800) Cholesterol sulfate is known to effect membrane stability and the present observations may help to explain the pathogenesis of STS deficiency and x-linked ichthyosis.


This article has been cited by other articles:


Home page
 CirculationHome page
H. Yanai, N. B. Javitt, Y. Higashi, H. Fuda, and C. A. Strott
Expression of Cholesterol Sulfotransferase (SULT2B1b) in Human Platelets
Circulation, January 6, 2004; 109(1): 92 - 96.
[Abstract] [Full Text] [PDF]

Home page
 J. Lipid Res.Home page
C. A. Strott and Y. Higashi
Cholesterol sulfate in human physiology: what's it all about?
J. Lipid Res., July 1, 2003; 44(7): 1268 - 1278.
[Abstract] [Full Text] [PDF]

Home page
 ScienceHome page
E. Epstein Jr, R. Krauss, and C. Shackleton
X-linked ichthyosis: increased blood cholesterol sulfate and electrophoretic mobility of low-density lipoprotein
Science, November 6, 1981; 214(4521): 659 - 660.
[Abstract] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Endocrinology Endocrine Reviews J. Clin. End. & Metab.
Molecular Endocrinology Recent Prog. Horm. Res. All Endocrine Journals
Copyright © 1981 by The Endocrine Society